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Pediatrics > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

Common types of soft tissue masses in children

A

Rhabdomyosarcoma
The botryoid variant (sarcoma botryoides), arising within the wall of the bladder or vagina, is seen almost exclusively in infants.

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2
Q

By what age do patients with sickle cell disease usually have functional asplenia

A

5yo

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3
Q

Who is at risk of acute splenic sequestration crisis and when does it go away

A

HbSS, HbS beta thal (S Beta-0), HbSC, HbSB+

Goes away when spleen atrophies in HbSS and HbSb0

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4
Q

how much blood to transfuse in Sickle cell crisis

A

3-5 ml/kg aliquots

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5
Q

low platelet, high mean platelet volume indicates

A

presence of autoantibodies

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6
Q

risk of administering anti-D immunoglobulin (in the setting of giving in ITP)

A

worsen anemia

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7
Q

Differential diagnosis in child with anemia and thrombocytopenia

A

leukemia

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8
Q

when to consider steroids in ITP

A

when leukemia is not on ddx, in older kids not at high risk of bleeding (because it takes a while to start working)

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9
Q

Causes of isolated neutropenia in infants (before 1yo)

A

Severe congenital neutropenia
Cyclic neutropenia (every 21d)

both are autosomal dominant

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10
Q

Treatment for febrile neutropenia other than antibiotics

A

G-CSF

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11
Q

most common childhood cancer

A

ALL/AML

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12
Q

When should you consider working up a child for leukemia?

A

unexplained fever, pain, pallor, fatigue, petechiae, bruising, and pathologic fractures

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13
Q

Specific tests for leukemia aside from CBC/BMP

A

LP
Flow cytometry
Boys: testicular exam

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14
Q

Factors for good prognosis in ALL (Age, WBC, CSF)

A

Children < 10 and >1 yo
WBC: <50,000
CSF not involved

No testicular disease

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15
Q

ALL treatment

A

Boys longer tx than girls (risk of testicular mass)

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16
Q

Syndromes associated with increased risk of ALL

A

trisomy 21
Klinefelter sd
Fanconi anemia
Bloom sd

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17
Q

When is prednisone urgently needed in ALL

A

mediastinal involvement causing respiratory compromised

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18
Q

Hemophilia A is a deficiency in factor ___

A

VIII

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19
Q

Most common congenital coagulation factor deficieny

A

hemophilia A

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20
Q

Female carriers of hemophilia A have symptoms of ___

A

menorrhagia

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21
Q

Level of factor VIII and risk classification

A

mild 6-30%
Mod 1-5%
Severe <1%

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22
Q

Triad of thrombocytopenia, eczema, and infections dx

A

wiskott-aldrich syndrome
X-linked disorder

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23
Q

How to diagnose HLH

A

an identified genetic mutation OR 5 of the following 8 criteria

  1. fever
  2. splenomegaly
  3. Cytopenia 2/3 blood cell lines
  4. Hypertriglyceridemia +/- hypofibrinogenemia
  5. Hemophagocytosis in spleen/bone marrow or lymph node without evidence of malignancy
  6. low or absent NK cell activity
  7. Elevated ferritin level
  8. elevated soluble CD25 (IL-2 receptor) level
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24
Q

Next steps after diagnosing HLH

A
  1. genetic testing for primary HLH
  2. Bone marrow aspirate and biopsy r/o malignancy and look for classic hemophagocytsis macrophage
  3. LP (see if intrathecal therapy is indicated(
  4. brain imaging to assess central nervous system disease
  5. eval of infectious cause of secondary HLH
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25
Q

Autoimmune hemolytic anemia dx

A

low Hb, nucleated RBC
high retic
IgG + DAT

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26
Q

Tx of AIHA

A

steroids

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27
Q

vitamin k is essential for factors ____

A

II, VII, IX, X

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28
Q

When does vitamin K deficiency bleeding occur

A

newborn to 6 mo of age

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29
Q

What’s the risk of severe VKDH and when does it happen

A

<1 week of age (early) an can involve intestinal or head bleed

30
Q

In TLS, what are the levels of potassium, phos, and uric acid?

A

all high

31
Q

where are craniopharyngiomas derived from?

A

pituitary embryonic tissue

32
Q

Sequelae of tx of craniopharyngiomas

A

pan hypopituitarism

risk of hypothalamic hyperphagia

33
Q

Tx of cranipharyngioma

A

Resection and radiation if not fully resected

34
Q

Risk of untreated brainstem glioma

A

herniation and resp failure

35
Q

Child with severe neutropenia comes to the ED, next steps

A

cultures and IV abx

36
Q

Neutropenia, is it innate or adaptive? What are you at risk of developing?

A

Innate

fungal and bacterial

37
Q

When to evaluate for immunodeficiencies (7)

A
  1. > 2 SYSTEMIC bacterial infections (sepsis, meningitis, osteomyelitis)
  2. > 2 serious respiratory infections (PNA)
  3. Multiple bacterial infections (cellulitis, draining otitis media, lynphadenitis)
  4. Unusual infections involving the liver or a brain abscess
  5. Infections by unusual pathogens (aspergillus, disseminated candidiasis, serratia, nocardia, bukholderia)
  6. Infections of unusual severity
  7. Chronic gingivitis or recurrent aphthous ulcers
38
Q

Hb electrophoresis for B thalassemia major

A

HbA 0*
HbA2 elevated
HbF +/-

39
Q

How to treat/cure beta thal major Hb B-0

A

blood transfusions
HSCT

40
Q

How to differentiate between iron deficiency and beta thal on mentzer index (MCV/RBC)

A

Iron deficiency >13
Beta thal <13

41
Q

Macrocytic anemia ddx

A

nutritional (B12/Folate)
Diamond Blackfan anemia

42
Q

Anemia due to destruction ddx

A

Intrinsic to RBC: hereditary spherocytosis, G6PD deficiency, sickle cell anemia, pyruvate kinase deficiency

Extrinsic to the RBC: antibody-mediated destruction (e.g. autoimmune hemolytic anemia), Hemolytic uremic syndrome (mechanical)

43
Q

Transfusion reactions (acute) and how to minimize

A

Febrile transfusion reaction (cytokine in donor plasma react to antigen on donor leukocyte) – leukoreduction

Allergic

Hemolytic

Bacterial contamination – should send blood product back to culture

Transfusion-associated graft vs host disease (donor lymphocutes recognize host as foreign) – irradiation

44
Q

When to refer bone pain to Oncology

A

lytic lesion

Periosteal reaction: Osteosarcoma, Ewing, Osteomyelitis

NO Periosteal reaction: Histiocytic disorder, neuroblastoma

45
Q

When to NOT refer patient to oncology for bone pain

A

solitary lesion
eccentric lytic destructive lesion and metaphyseal expansion (non-neoplastic)

DO get CT if it is a sharply marginated radiolucent lesion. May be osteochondroma, osteoid osteoma, osteoblastoma, fibroma

46
Q

How to treat pain from osteoid osteoma

A

NSAID

Worsens at end of day, not related to activity

Resect lesion

47
Q

Boils, recurrent pneumonia, recurrent bacterial infections/abscesses. What do you suspect is the issue?

What if there are family members who have it?

A

Innate immune system - CHRONIC GRANULOMATOUS DISEASE (CGD)

  • issue with oxidative radicals

Neutrophils, monocytes, NK cells

X-linked (all males)
Mutation CYBB

48
Q

How do you test for CBD/ oxidative burst?

A

Flow cytometric assessment of oxidative burst

  • dihydroxy-rhodamine123 reduction
49
Q

Mutations in ELANE causes ___

A

severe congenital neutropenia

50
Q

What causes HbSS mutation

A

Replacement of glutamic acid with valine at position 6 of B-globulin

51
Q

How to diagnose adrenal mass in neonates/prenatally identified adrenal mass

A

urine vanillymandelic acid (VMA)
homovanillic acid (HVA)

or positive metaiodobenzylguanidine (MIBG) scan
likely neuroblastoma

52
Q

most common extracranial solid tumor in children

A

neuroblastoma

53
Q

Factors that are favorable for neuroblastoma

A

< 18mo
Lower stage
NO MYCN amplification
DNA index that is not diploid
Heterozygosity at 1p and 11q

54
Q

Management of neonatal neuroblastoma

A

reassurance and periodic US

should self-resolve

55
Q

Serum marker for hepatoblastoma

A

alpha fetoprotein

56
Q

Serum marker for germ cell tumor

A

HCG

57
Q

Where can neuroblastoma show up

A

Adrenals
Paraspinal ganglia (near the spine)

58
Q

Triad of botulinum Toxin

A

bulbar palsy
Descending paralysis
Clear sensorium

59
Q

If you see pancytopenia, next step?

A

Bone marrow biopsy

60
Q

Differential for pancytopenia – hypoproliferation (6)

A

Leukemia
Aplastic anemia
Nutritional deficiency
Metastatic cancer infiltrating bone marrow (neuroblastoma/rhabdomyosarcoma)
Fulminant sepsis
MDS

61
Q

Differential for pancytopenia – increased destruction (3)

A

Splenomegaly
Paroxysmal Nocturnal Hemoglobinuria (complement mediated)
Acquired hemophagocytic lymphohistiocytosis

62
Q

Normal MVCs by age (roughly)

A

Higher than adult range as newborns - 3d >95

2wk >86
2mo >77
3-6 mo >74
NADIR 2-6 yo >70

63
Q

Side effect of etoposide

A

AML
MDS

64
Q

Side effect of daunorubicin (anthracycline)

A

cardiomyopathy

65
Q

Side effects of cytarabene and cyclophosphamide

A

gonadal failure, bladder cancer

66
Q

Side effects of cytarabene and cyclophosphamide

A

gonadal failure, bladder cancer

67
Q

At what serum lead level should a child be started on iron supplementation

A

> 5

68
Q

Anticholinergic toxidrome

A

mad as a hatter
dry as a bone
hot as a hare
red as a beet
blind as a bat (pupillary dilation)

69
Q

Anticholinergic toxidrome

A

mad as a hatter
dry as a bone
hot as a hare
red as a beet
blind as a bat (pupillary dilation)

70
Q

Tx for acute methanol toxicity

A

fomepizole

Pediatrics (26 decks)

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